The role of microvascular injury in the evolution of idiopathic pulmonary fibrosis
The role of microvascular injury in the evolution of idiopathic pulmonary fibrosis
复制标题
DOI：
10.1309/0b06y93ege6tq36y
复制
发表时间：
2003-04-01
影响因子：
3.5
通讯作者：
Ross, P
中科院分区：
文献类型：
--
作者：
Magro, CM;Allen, J;Ross, P
19例既往健康患者并发特发性肺纤维化（IPF）的间质性肺病虽然所有患者均存在间质性和/或蜂窝状实质纤维化，但也有斑片状的少细胞性间隔毛细血管损伤区，同时也有体液介导的微血管损伤综合征的直接免疫荧光证据。在接受检测的18名患者中，有17名患者的因子VIII水平显著升高。所有18例患者均存在抗磷脂，包括磷脂酰乙醇胺、β -2糖蛋白、磷脂酰胆碱和/或磷脂酰丝氨酸的抗体。4例患者出现抗ro和抗核糖核蛋白（RNP）抗体。血清学证据显示9例患者感染巨细胞病毒（CMV）， 9例感染细小病毒B19 (B19)；我的病人没有接受检查。分子研究显示6例血清B19阳性患者中有6例存在B19 DNA。原位杂交研究显示，尽管没有CMV感染典型的细胞病变，但有活动性CMV感染血清学证据的患者肺细胞中存在CMV RNA。与B19和巨细胞病毒相关的抗磷脂抗体、抗内皮细胞抗体和嗜内皮病毒感染可能对IPE的演变具有重要的病理意义。
Interstitial lung disease compatible with idiopathicpulmonary fibrosis (IPF) developed in 19 previously healthy patients. Although interstitial and/or honeycomb parenchymal fibrosis was present in all, there were patchy areas of paucicellular septal capillary injury along with corroborative direct immunofluorescent evidence of a humorally mediated microvascular injury syndrome. Significantly elevated factor VIII levels were seen in 17 of 18 patients tested. Antiphospholipids were present in all 18 patients tested, comprising antibodies of phosphatidylethanolamine, beta-2 glycoprotein, phosphatidylcholine, and/or phosphatidylserine. Anti-Ro andlor anti-ribonucleoprotein (RNP) antibodies were seen in 4 patients. Serologic evidence of infection with cytomegalovirus (CMV) was found in 9 patients and parvovirus B19 (B19) in 9 patients; I patient was not tested. Molecular studies revealed B19 DNA in 6 of 6 B19-seropositive patients. In situ hybridization studies revealed CMV RNA in pulmonary cells inpatients with serologic evidence of active CMV infection despite the absence of cytopathic changes typical of CMV infection.Antiphospholipid antibodies, antiendothelial cell antibodies, andlor endotheliotropic viral infections related to B19 and CMV may be of pathogenetic importance to the evolution of IPE This report underscores the potential importance of microvascular injury in the evolution of IPF.